慢性粒-单核细胞白血病合并结外非霍奇金淋巴瘤的临床特征分析

Chronic myelomonocytic leukemia accompanied with extranodal lymphoma : a clinical feature analysis

目的 :探讨慢性粒-单核细胞白血病(chronic myelomonocytic leukemia,CMML)合并结外非霍奇金淋巴瘤(non-Hodgkin's lymphoma,NHL)患者的临床特征及其鉴别诊断。方法 :回顾性分析2例CMML合并结外NHL患者的临床资料、影像学检查、病理形态学及免疫组织化学(免疫组化)标志特征,并复习国内外相关文献进行探讨。结果:2例CMML合并结外NHL患者均为老年女性,因乏力或出现相应部位肿瘤侵犯症状就诊。例1患者诊断为CMML后发生NHL,例2患者则同时诊断为CMML和NHL。实验室检查示,2例患者的外周血白细胞计数增高,以单核细胞为主;乳酸脱氢酶升高;骨髓增生活跃伴有病态造血;细胞免疫表型为CD14+CD64+;染色体荧光原位杂交分析BCR-ABL为阴性(-)。PET/CT检查显示2例患者均未发现骨髓高代谢灶,例1患者表现为小肠异常高代谢,最大标准化摄取值(maximum standard uptake values,SUVmax)值为12.60;例2患者表现为肺内异常高代谢,SUVmax值为6.72。病理检查提示2例患者均为B细胞来源的NHL;免疫组化检查结果提示,LCA、CD20、CD79a、Bcl-6、Ki-67均为阳性,CD5、CD10、CD3、Cylin D1、CD56、CD23、CD21阴性。结论:CMML合并结外NHL罕见,患者缺乏特异性的临床表现,而结合实验室、影像学检查及组织病理、免疫组化检查进行分析,可提高其诊断的准确率。

Objective： To investigate the clinical characteristics and differential diagnosis of chronic myelomonocytic leukemia（CMML） accompanied with non-Hodgkin ＇s lymphoma（NHL）. Methods： The clinical data, imaging findings,pathomorphological features and immunohistochemical markers of two patients with CMML accompanied with NHL were retrospectively analyzed, and related literatures were reviewed. Results： The 2 cases were elderly women presented with fatigue or symptoms of tumor invasion at corresponding sites. Case 1 was diagnosed with CMML and thereafter having NHL diagnosed, and case 2 was diagnosed with CMML and NHL simultaneously. Laboratory examination showed that pe-ripheral white blood cells were increased,especially mononuclear cells, and lactate dehydrogenase was also increased.Bone marrow smears showed active proliferation with morbid hematopoiesis. On immunophenotyping, the bone marrow cells often expressed CD14＋CD64＋. Analysis of chromosome fluorescence in situ hybridization showed that BCR-ABL fusion gene were negative. PET/CT examination displayed that high metabolic lesions in bone marrow were not found in the2 cases, but showed hypermetabolic small intestinal lesions in case 1 and lung lesions in case 2. SUVmax value were12.60 and 6.72, respectively. Pathological examination suggested that these 2 patients were B-cell-derived NHL. Immunohistochemical study showed that LCA, CD20, CD79 a, Bcl-6 and Ki-67 were positive, CD5, CD10, CD3, Cylin D1, CD56,CD23 and CD21 were negative. Conclusions： CMML accompanied with NHL is rare and lacks specific clinical manifestation. With the laboratory findings combined with imaging, histopathology and immunohistochemical examination, the diagnostic accuracy could be improved.